Fontan-Associated Liver Disease

Daniels CJ, Bradley EA, Aboulhosn J, et al.
Fontan-Associated Liver Disease: Proceedings From the American College of Cardiology Stakeholders Meeting, October 1 to 2, 2015, Washington DC. J Am Coll Cardiol 2017;Dec 18:[Epub ahead of print].

The following are key points to remember from this American College of Cardiology stakeholders conference report on Fontan-associated liver disease (FALD):

  1. FALD is a noncardiac complication of the Fontan circulation, which has been recognized with increasing frequency in adolescents and adults.
  2. The etiology of FALD is not entirely known, but is likely multifactorial, with physiological derangements, particularly elevation in central venous pressures, medical complications, and surgical interventions likely contributing to liver pathology.
  3. Abnormal liver histology is present in nearly all patients after Fontan surgery. These findings range from evidence of fibrosis to cirrhotic changes.
  4. Risk factors for FALD include higher Fontan pressures, increasing age, longer duration of Fontan, underlying hepatitis B or C, alcohol use, and hepatotoxic drug use.
  5. Prevention of FALD is an important consideration. This can be attempted by optimization of anatomy and physiology, as well as prevention of liver injury both prior to and after the Fontan. Examples of prevention of liver injury include an aggressive approach towards immunization against and treatment of viral hepatitis, as well as avoidance of hepatoxins (safe amount of alcohol unknown) and obesity (steatohepatitis).
  6. The committee recommends that children with Fontan circulation undergo baseline measures of liver function 5 years after the Fontan operation, or sooner if there is a clinical suspicion of liver injury or Fontan failure. Studies should be repeated no less than every 3-5 years in childhood. Adolescents and adults should have evaluation of liver function every 1-2 years.
  7. Baseline laboratory studies should include aspartate aminotransferase (AST), alanine aminotransferase (ALT), gamma-glutamyltransferase (GGT), alkaline phosphatase, albumin, total protein, international normalized ratio (INR), complete blood cell count, and platelets. Additional screening should include hepatitis C antibody and hepatitis A and B immunoglobulin G titers.
  8. Abdominal ultrasound should be performed at baseline with consideration for elastography. Abnormal liver imaging is common and can range from changes of mild congestive hepatopathy to changes of advanced fibrosis.
  9. Patients with abnormal screening tests should be referred to hepatology for further evaluation and management. Examples of abnormal evaluation include thrombocytopenia, prolonged prothrombin time in the absence of anticoagulation, abnormal liver enzymes greater than twice the upper limit of normal, elevated direct bilirubin, hypoalbuminemia, splenomegaly, or liver mass.
  10. Treatment of FALD (patients with cirrhosis or stage III/IV fibrosis) is based on optimization of the Fontan circulation and hepatology consultation. Testing serum alpha-fetoprotein and liver imaging every 6 months should be considered because of increased risk of hepatocellular carcinoma.
  11. Patients with imaging evidence of cirrhosis should be referred to hepatology for consideration of biopsy if it will change clinical management. Biopsy is not necessary in patients with clinical evidence of cirrhosis (esophageal varices, marked splenomegaly, hepatic encephalopathy, or platelets <70,000).
  12. The committee recommended multiple action items, including partnering with the Centers for Disease Control to determine the incidence and prevalence of FALD; registry development; development of multicenter studies; better understanding of heart failure and transplant issues around FALD; and engaging patient organizations, patients, and families to advocate for further resources to be dedicated to Fontan patients and FALD.

Keywords: Carcinoma, Hepatocellular, Cardiac Surgical Procedures, Diagnostic Imaging, Fatty Liver, Fontan Procedure, Heart Defects, Congenital, Heart Failure, Hepatitis, Hypoalbuminemia, Liver Cirrhosis, Liver Diseases, Obesity, Secondary Prevention, Splenomegaly, Thrombocytopenia, Transplantation

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